National Cancer Institute

at the National Institutes of Health

Paranasal Sinus and Nasal Cavity Cancer Treatment (PDQ®)

Health Professional Version

Table of Contents

General Information About Paranasal Sinus and Nasal Cavity Cancer

Incidence and Mortality

The majority of tumors of the paranasal sinuses present with advanced disease,
and cure rates are generally poor (≤50%). Squamous cell carcinoma (SCC) is the most frequent
type of malignant tumor in the nose and paranasal sinuses (70%–80%).
Papillomas are distinct entities that may undergo malignant degeneration. The
cancers grow within the bony confines of the sinuses and are often asymptomatic
until they erode and invade adjacent structures.[1-3]

Nodal involvement is
infrequent. Although metastases from both the nasal cavity and paranasal sinuses may occur, and distant metastases are found in 20% to 40% of
patients who do not respond to treatment, locoregional recurrence accounts for the majority of cancer deaths since most patients die of direct extension into vital areas of the skull or of
rapidly recurring local disease.

Cancers of the maxillary sinus are the most common of the paranasal sinus
cancers. Tumors of the ethmoid sinuses, nasal vestibule, and nasal cavity are
less common, and tumors of the sphenoid and frontal sinuses are rare.

Anatomy

The major lymphatic drainage route of the maxillary antrum is through the
lateral and inferior collecting trunks to the first station submandibular,
parotid, and jugulodigastric nodes and through the superoposterior trunk to
retropharyngeal and jugular nodes.

Clinical Evaluation and Follow-up

Pretreatment evaluation and staging, as well as the need for
multidisciplinary planning of treatment, is very important. Generally, the
first opportunity to treat patients with head and neck cancers is the most
effective, though occasionally salvage surgery or salvage radiation therapy,
as appropriate, may be successful. Since most treatment failures occur
within 2 years, the follow-up of patients must be frequent and meticulous
during this period. In addition, because nearly 33% of these patients
develop second primary cancers in the aerodigestive tract, a lifetime of
follow-up is essential.

Carcinogenesis and Risk Factors

Some data indicate that various industrial exposures may be related to cancer
of the paranasal sinus and nasal cavity. The risk of a second primary head and
neck tumor is considerably increased.[4] A subgroup has shown that paranasal sinus and nasal cavity SCC are associated with human papilloma virus (HPV) infection and that HPV-positive patients may have a better prognosis than those who are HPV-negative.[5]

Cellular Classification of Paranasal Sinus and Nasal Cavity Cancer

The most common cell type for paranasal sinus and nasal cavity cancers is
squamous cell carcinoma. Minor salivary gland tumors comprise 10% to 15% of
these neoplasms. Malignant melanoma presents in <1% of neoplasms in
this region. Some 5% of cases are malignant lymphomas.[1,2]

Stage Information for Paranasal Sinus and Nasal Cavity Cancer

The staging systems are clinical estimates of the extent of disease. The
assessment of the tumor is based on inspection, palpation, and direct endoscopy
when necessary. The tumor must be confirmed histologically, and any other
pathological data obtained on biopsy may be included. The appropriate nodal
drainage areas are examined by careful palpation. Computed tomographic and/or
magnetic resonance imaging studies are generally required to adequately
evaluate tumor extent prior to attempted surgical resection or definitive
radiation therapy. If a patient relapses, complete restaging must be done to select the appropriate additional therapy.[1,2]

Definitions of TNM

Staging of nasal cavity and paranasal sinus carcinomas is not as well
established as for other head and neck tumors. For cancer of the maxillary sinus, the nasal cavity, and the ethmoid sinus, the
American Joint Committee on Cancer (AJCC) has designated staging by TNM classification.[3]

Treatment Option Overview

Except for T1 mucosal carcinomas, the accepted method of treatment is a
combination of radiation therapy and surgery. The incidence of lymph node
metastases is generally low (approximately 20% of all cases). Thus, routine
radical neck dissection or elective neck radiation therapy is recommended only for
patients presenting with positive nodes.

For patients with operable tumors,
radical surgery is generally performed first to remove the bulk of the tumor
and to establish drainage of the affected sinus(es). This is followed by
postoperative radiation therapy. Some institutions continue to give a full
dose of radiation therapy preoperatively for all stage II and stage III tumors and
to operate 4 to 6 weeks later.[1-3] A review of published clinical results of
radical radiation therapy for head and neck cancer suggests a significant loss
of local control when the administration of radiation therapy was prolonged;
therefore, lengthening of standard treatment schedules should be avoided
whenever possible.[4]

Surgery

Surgical exploration may be required to determine operability.

Destruction of
the base of skull (i.e., anterior cranial fossa), cavernous sinus, or the pterygoid
process; infiltration of the mucous membranes of the nasopharynx; or
nonresectable lymph node metastases are relative contraindications to surgery.
Surgical approaches include fenestration with removal of the bulk tumor, which
is usually followed by radiation therapy or block resection of the upper jaw.
A combined craniofacial approach, including resection of the floor of the
anterior cranial fossa is used with success in selected patients.[5] Removal of
the eye is performed if the orbit is extensively invaded by cancer. Clinically
positive nodes, if resectable, may be treated with radical neck dissection.

Radiation Therapy

Radiation therapy must be carried to high doses for any significant probability
of permanent control. The treatment volume must include all of the maxillary
antrum and involved hemiparanasal sinus and contiguous areas. The orbit and
its contents are excluded except under unusual circumstances. Lymph nodes of
the neck, when palpable, should be treated in conjunction with treatment of
advanced carcinomas of the antrum. This may be unnecessary for early tumors.

Accumulating evidence has demonstrated a high incidence (>30%–40%) of
hypothyroidism in patients who have received external-beam radiation therapy to the
entire thyroid gland or to the pituitary gland. Thyroid function testing of
patients should be considered prior to therapy and as part of posttreatment
follow-up.[6,7]

Recurrent Disease

For patients with recurrent disease, chemotherapy trials should be considered.
Chemotherapy for recurrent squamous cell cancer of the head and neck has been
shown to be efficacious as palliation and may improve quality of life and
length of survival. Various drug combinations including cisplatin,
fluorouracil, and methotrexate are effective.[8,9]

Treatment of tumors of the paranasal sinuses and of the nasal cavity should be
planned on an individual basis because of the complexity involved.

Surgery plus radiation therapy for tumors of the septal and lateral
walls.[5]

For inverting papilloma:

Surgical excision.

Re-excision for surgery failures.

Radical surgery may eventually be necessary.

Radiation has been used successfully for surgical failures.

For melanomas and sarcomas:

Surgical excision if possible.

Combined surgery, radiation, and chemotherapy are recommended for
rhabdomyosarcoma.

For midline granuloma:

Radiation therapy to nasal cavity and paranasal sinuses.

For nasal vestibule tumors:

Surgery or radiation may be performed. If lesions are extremely small,
surgery is preferred provided that no deformity is expected and a need
for reconstruction is not anticipated. Radiation therapy is preferred
for other small lesions.[6,7] Treatment of the ipsilateral neck should be considered.

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with
stage I paranasal sinus and nasal cavity cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

For nasal cavity tumors (squamous cell carcinomas), treatment preferences
are either surgery or radiation therapy, which have equal cure rates:[4]

Surgery or radiation therapy for tumors of the septum.

Radiation therapy for tumors of the lateral and superior walls.
Concomitant chemotherapy and radiation therapy may be considered.

Surgery plus radiation therapy for tumors of the septal and lateral
walls.[5]

For inverting papilloma:

Surgical excision.

Re-excision for surgery failures.

Radiation therapy for radical surgery failures may eventually be
necessary.

For melanomas and sarcomas:

Surgical excision if possible.

Combined surgery, radiation, and chemotherapy are recommended for
rhabdomyosarcoma.

For midline granuloma:

Radiation therapy to nasal cavity and paranasal sinuses.

For nasal vestibule tumors:

Surgery or radiation therapy may be performed. If tumors are extremely
small, surgery is preferred provided that no deformity is expected and
a need for reconstruction is not anticipated. Radiation therapy is
preferred for other small lesions.[6,7] Treatment of the neck should
be considered.

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with
stage II paranasal sinus and nasal cavity cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

Clinical trials using new drug combinations for advanced tumors should be
considered to evaluate chemotherapy preoperatively or before radiation therapy, or as adjuvant therapy after surgery or after combined modality
therapy.

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with
stage III paranasal sinus and nasal cavity cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

Stage IV Paranasal Sinus and Nasal Cavity Cancer

Stage IV disease includes advanced lesions.

Standard treatment options:

For maxillary sinus tumors:

High-dose radiation therapy is used because extension to base of skull and nasopharynx is
a potential, but not absolute, contraindication to surgery. If radiation therapy is to be used alone, localized
drainage of the sinus(es) must be established before initiating radiation
therapy treatments.

Appropriate radiation and various chemotherapy agents should be
considered.

For midline granuloma:

Radiation therapy to nasal cavity and paranasal sinuses.

For nasal vestibule tumors:

Generally, radiation is preferred to minimize deformity. External-beam (i.e., photons or electrons) and/or interstitial implantation can be
used. Surgery is reserved for salvage. Treatment of the neck should
be considered.

Clinical trials for advanced tumors should be
considered to evaluate chemotherapy preoperatively or before radiation therapy,
as is adjuvant therapy after surgery or after combined modality therapy.

Concomitant chemotherapy and radiation therapy may be considered.

Neoadjuvant chemotherapy as employed in clinical trials has been used to shrink
tumors and to render them more definitively treatable with either surgery
or radiation. This chemotherapy is given prior to the other modalities; therefore,
the designation of neoadjuvant is used to distinguish it from standard adjuvant therapy,
which is given after or during definitive therapy with radiation or after
surgery. Many drug combinations have been used in neoadjuvant
chemotherapy.[6-8]

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with
stage IV paranasal sinus and nasal cavity cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

Recurrent Paranasal Sinus and Nasal Cavity Cancer

Chemotherapy for recurrent head and neck squamous cell cancer has shown
promise. Chemotherapy may be indicated where there is recurrence in either
distant or local disease after primary surgery or radiation, and when there is
residual disease after primary treatment.[1,2] Survival may be improved in
those achieving a complete response to chemotherapy.[3] Combined modality
therapy with platinum and radiation therapy has been used in trials such as UMCC-8810.[4]

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with
recurrent paranasal sinus and nasal cavity cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

About This PDQ Summary

Purpose of This Summary

This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of paranasal sinus and nasal cavity cancer. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

be discussed at a meeting,

be cited with text, or

replace or update an existing article that is already cited.

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Paranasal Sinus and Nasal Cavity Cancer Treatment are:

Scharukh Jalisi, MD, FACS (Boston University Medical Center)

Eva Szabo, MD (National Cancer Institute)

Minh Tam Truong, MD (Boston University Medical Center)

Any comments or questions about the summary content should be submitted to Cancer.gov through the Web site's Contact Form. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.

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